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目的:探讨成熟NK细胞肿瘤的临床特点、诊断及治疗效果。方法:收集14例成熟NK细胞肿瘤病例并进行回顾性分析。结果:侵袭性NK细胞白血病(ANKL)4例,结外NK细胞淋巴瘤鼻型(ENKL)10例。4例ANKL病情呈暴发性,进展快,临床表现为发热,肝脾肿大,贫血,多脏器功能衰竭,均治疗无效死亡。10例ENKL临床特点为局部肿块,溃疡坏死伴出血或脓性分泌物。5例死亡,5例正在治疗中。结论:成熟NK细胞肿瘤较罕见,目前对其发病机制不明,ANKL诊断主要依据临床表现、骨髓细胞学检查和免疫学分型,采用急性淋巴细胞白血病的化疗方案治疗。ENKL诊断主要依据临床表现、组织活检和免疫组化,采用化放疗联合治疗。
Objective: To investigate the clinical features, diagnosis and treatment of mature NK cell tumors. Methods: Totally 14 cases of mature NK cell tumors were collected and analyzed retrospectively. Results: There were 4 cases of aggressive NK cell leukemia (ANKL) and 10 cases of extranodal NK cell lymphoma (ENKL). 4 cases of ANKL condition was fulminant, rapid progress, clinical manifestations of fever, hepatosplenomegaly, anemia, multiple organ failure, were ineffective treatment of death. Ten cases of ENKL clinical features of local mass, ulcer necrosis with bleeding or purulent discharge. Five patients died and five were under treatment. Conclusions: Mature NK cell tumors are relatively rare and their pathogenesis is unknown at present. The diagnosis of ANKL is mainly based on the clinical manifestations, myelocytology and immunological typing, and the chemotherapy regimen of acute lymphoblastic leukemia. ENKL diagnosis mainly based on clinical manifestations, biopsy and immunohistochemistry, the use of combined radiotherapy and chemotherapy.